Atopic keratoconjunctivitis

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2Active trials3Specialists8Treatment centers

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UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
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Overview

Atopic keratoconjunctivitis (AKC) is a chronic, bilateral inflammatory condition of the eyes that occurs in association with atopic dermatitis (eczema). It is considered one of the most severe forms of allergic eye disease and primarily affects the conjunctiva (the membrane lining the eyelids and covering the white of the eye) and the cornea. AKC typically develops in individuals who have a history of systemic atopic disease, including atopic dermatitis, asthma, or allergic rhinitis. The condition is driven by a complex interplay of IgE-mediated and T-cell-mediated immune responses. Key symptoms include intense itching, burning, tearing, and mucoid or ropy discharge from the eyes. Patients often experience significant eyelid involvement with thickening, scaling, and crusting of the lid margins (blepharitis). The conjunctiva may show papillary hypertrophy, scarring, and fibrosis, particularly of the tarsal (inner eyelid) conjunctiva. Corneal complications are common and can be sight-threatening, including punctate epithelial erosions, corneal neovascularization, ulceration, scarring, and keratoconus. Patients are also at increased risk for cataracts (particularly anterior subcapsular cataracts), herpes simplex keratitis, and secondary bacterial infections such as staphylococcal blepharitis. Treatment of AKC involves a multimodal approach. First-line therapies include avoidance of known allergens, cold compresses, and preservative-free artificial tears for lubrication. Topical antihistamines and mast cell stabilizers (such as olopatadine) are commonly used. For moderate to severe disease, topical corticosteroids may be required for acute flares, though long-term use is limited by the risk of glaucoma and cataracts. Topical calcineurin inhibitors such as tacrolimus and cyclosporine have become important steroid-sparing agents for chronic management. Systemic immunosuppressive therapy or biologic agents (such as dupilumab) may be considered in refractory cases. Management of concurrent atopic dermatitis and lid hygiene are essential components of care. Despite treatment, AKC can lead to progressive visual impairment if corneal complications are not adequately controlled.

Clinical phenotype terms— hover any for plain English:

BlepharitisHP:0000498Allergic conjunctivitisHP:0007879Loss of eyelashesHP:0011457Corneal neovascularizationHP:0011496Conjunctival hyperemiaHP:0030953Corneal scarringHP:0000559ChemosisHP:0012375
Inheritance

Multifactorial

Caused by a mix of several genes and environmental factors

Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗NORD ↗

FDA & Trial Timeline

2 events
Dec 2025An Efficacy and Safety Study of DFL24498 in the Treatment of AKC

Dompé Farmaceutici S.p.A — PHASE3

TrialRECRUITING
Aug 2021Ocular Comorbidity in Atopic Dermatitis

Rigshospitalet, Denmark

TrialACTIVE NOT RECRUITING

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Treatments

No FDA-approved treatments are currently listed for Atopic keratoconjunctivitis.

2 clinical trialsare actively recruiting — trials can provide access to cutting-edge therapies.

View clinical trials →

Clinical Trials

2 recruitingView all trials with filters →
Phase 31 trial
An Efficacy and Safety Study of DFL24498 in the Treatment of AKC
Phase 3
Actively Recruiting
· Sites: Jacksonville, Florida; Miami, Florida +17 more · Age: 1865 yrs
Other1 trial
Ocular Comorbidity in Atopic Dermatitis
Active
PI: Steffen Heegaard, MD, DMSci (Department of Ophthalmology, Rigshospitalet-Glostr) · Sites: Glostrup Municipality

Specialists

3 foundView all specialists →
QM
Quan D Nguyen, MD, MSc
Specialist
PI on 5 active trials
EM
Esen K Akpek, MD
BALTIMORE, MD
Specialist
PI on 2 active trials
SD
Steffen Heegaard, MD, DMSci
Specialist
PI on 1 active trial

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Atopic keratoconjunctivitis.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Atopic keratoconjunctivitis

Disease timeline:

New recruiting trial: An Efficacy and Safety Study of DFL24498 in the Treatment of AKC

A new clinical trial is recruiting patients for Atopic keratoconjunctivitis

Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

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Family & Caregiver Grants

Financial assistance programs specifically for caregivers of rare disease patients.

Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

Common questions about Atopic keratoconjunctivitis

What is Atopic keratoconjunctivitis?

Atopic keratoconjunctivitis (AKC) is a chronic, bilateral inflammatory condition of the eyes that occurs in association with atopic dermatitis (eczema). It is considered one of the most severe forms of allergic eye disease and primarily affects the conjunctiva (the membrane lining the eyelids and covering the white of the eye) and the cornea. AKC typically develops in individuals who have a history of systemic atopic disease, including atopic dermatitis, asthma, or allergic rhinitis. The condition is driven by a complex interplay of IgE-mediated and T-cell-mediated immune responses. Key sympt

How is Atopic keratoconjunctivitis inherited?

Atopic keratoconjunctivitis follows a multifactorial inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Atopic keratoconjunctivitis typically begin?

Typical onset of Atopic keratoconjunctivitis is adult. Age of onset can vary across affected individuals.

Are there clinical trials for Atopic keratoconjunctivitis?

Yes — 2 recruiting clinical trials are currently listed for Atopic keratoconjunctivitis on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.

Which specialists treat Atopic keratoconjunctivitis?

3 specialists and care centers treating Atopic keratoconjunctivitis are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.